CRMS and Culture results

[emason]

My 1 year old son has a CRMS dx. He just had a clinic appointment a week and a half ago which resulted in a second negative sweat. Our pulm said at the appt that he appears to be healthy - clear lungs etc. She called yesterday to tell me that his culture results came back positive for staph, Chryseobacterium Indologenes, and Acinetobacter Baumannii but that because he is appearing healthy and has a negative sweat that they are nothing to be concerned about.

Today he started having a running nose. He's 12.5 months so my first thought was teething. But as the night has progressed he has been seeming to be having some respiratory symptoms. It's 1230 am and I'm listening to (and watching him) him on the monitor. He is still asleep but I am hearing some wheezing. I guess my question is really related to his culture in combination with the wheezing. His culture was 1.5 weeks ago, at which time he had NO cough, no wheezing, nothing of any concern. At a week and a half post culture, he is now appearing to have some symptoms of a respiratory illness. Because he is CRMS and not CF, it's more complicated - they do not treat him as if he as CF, they treat him as if he is healthy unless proven otherwise.

My question is really just what would you think about a child presenting with respiratory symptoms 1.5 weeks post a positive culture? Should I notify his clinic? Or is this most likely just a case of bad luck? In all honesty it sounds like early croup - he's had 3 bouts of croup since December.. I don't know if that is in line with something that coupled with a positive culture for the 3 things listed about or if I'm just being a paranoid mother. Any insight is appreciated.

 

[Aboveallislove]

Hey Mom,

So sorry for the long and worry-filled night! I would definitely call the CF clinic, either the on-call doctor today if your little one is seeming to get worse, or first thing Monday morning, explain what's going on and they can guide you.

A few thoughts I have, coming from the backdrop of I'm not a fan of the CRMS diagnosis. I understand why they do it, but if the genes are mutated, even if the sweat test isn't positive, the genes likely aren't working as they should. And I'm also a more proactive type and I fear that that diagnosis leads doctors not to be proactive in some cases where they should. Given that your little one has cultured 3 bugs and that that is not typical for "normal" kids, to me, that says that the genes not working right are causing issues even if the lungs sound clear. And 3 bouts of croup also sounds like a red flag for the need of something more proactive. With CF the mucus is thicker so it's harder to clear the virus and they have a thriving ground to grow in all the mucus. Now, it might be they don't want to do antibiotics, although if she is starting to cough more with those 3 bugs, that would seem a good option, the doctors can explain their thoughts and reasons. But at a minimum, it would make sense to do a brochial dilator (Albuterol or Xopenex--Xopenex has less jiterryiness and is now generic too so if they do script Albuterol you might ask if they can give you Xopenex instead), as well as some percussions. That will help he breathe more easily.

I also think it might depend on what cause the CRMS diagnosis--does she have one known CF causing mutation and one NOT believed to cause CF? Or is the second gene one that is unknown whether it does, or one that may or may not. If it is anything other than the one known and one NOT believed to cause CF, personally, I'd push for a more proactive approach because the jury's still out and in a few years they might say that second mutation is disease causing and that's lost time to keep the lungs healthy. Obviously, these are just my thoughts and other's might have different one and you need to discuss with the doctor to see what they think based on their expertise and pros/cons of the approaches.

Good luck Mom.

 

[emason]

I have the same concerns about his CRMS dx as you have listed. Our clinic does not do any preventative treatments for CRMS children unless they are symptomatic. The pulm reminds me every time we have a conversation that my son does not have CF (for example she will say things like, "yes he has all these things culturing but normal people culture stuff too and your son does not have CF!" At our clinic everything revolves around the sweat test (which I think is pretty common) and my son has passed 2 sweat tests with flying colors so we just aren't high on their concern list. On one hand I don't want to subject him to treatments he doesn't need but on the other (much bigger) hand I don't want to look back at this time and wish I'd done more for him.

His second mutation is quite rare (g1069r - coupled with df508) - it is considered a mutation of "varying consequence" so one that sometimes does, sometimes doesn't. It's a little strange because according to the CFTR database it usually presents with low sweats but is associated with Pancreatic Insufficiency - so our pulm has said she thinks he's more likely to have pancreatic issues than lung. From my perspective he's had a lot of red flags - this is now his 11th respiratory illnesses since October. But the clinic isn't seeming overly concerned and just says that some kids (even healthy kids) get sick a lot.

He's still wheezing this morning and he has what sounds like the croup bark starting up so I'm almost positive this is now his 4th croup since Christmas (sounds like a country song). Every time he's had croup he's had some mild respiratory distress so after his 3rd croup our ped prescribed him a nubulizer (albuterol) and a prednisone to have on hand in the event that he had another bout. I'm about to get familiar with it now because he clearly needs a little help opening him up.

Thanks for taking the time to reply and the advice! I'm supposed to be in touch with the pulmonologist this week anyways as we are awaiting the results of his latest fecal elastase test. I hate to admit this but I am almost positive that if I call and leave a message for her about him being sick she won't call back - she's been pretty clear that she thinks he's just having bad luck by being sick all the time. My husband and I are seriously considering switching clinics because of how marginalized we feel there. He had an ER visit in May from his last croup (also had a sinus infection) and they thought he might have pneumonia so they did a chest x-ray. They cleared him for pneumonia but called me the next day to say that the xray showed infiltrates. Of course that concerned me so I tried to get our pulmonolgist to weigh in and I never received any replies. So I'm sure a case of croup won't even get a nod. But she does like to discuss testing results one on one so I'll make sure to use that as a way to get the conversation in.

 

[imported_Momto2]

I would switch clinics. You need to trust that these people are batting for your son, will take your concerns seriously, and take the time to explain things to you clearly. If he has one df508 and another that is considered varying consequence, I would think they would take any GI or pulmonary symptoms seriously. Eleven respiratory illnesses in 9 months is a lot. I have noticed that my parents, who are just carriers of the d508, but have had more than their share of pulmonary and GI issues over the years. If it were my child, I would go where I felt I was being heard. Your son may very well need some of the meds used to treat CF to help him stay healthy.

 

[Ratatosk]

I too have a real tough time with the crms diagnosis and lack of treatment. Imo 2 mutations equal cf. my own child has two mutations, yet early on, had a normal sweat test of 32. Early on I took him to the clinic for a horrible cough and was told "they cough, it's what they do". He had bronchitis. I suggest asking/telling the doctor to treat the symptoms. Cpt to clear those lungs, beat that cough, wheeze out of him. If the doctor declines, I'd say, there's something wrong, please put in writing in my sons chart that you declined to treat this and then find a new clinic.

 

[Gammaw]

You've received great advice here and I won't repeat it for you. But I really want to emphasize one thing. A clinic that refuses to call you back is the wrong clinic. No matter what they think, they should have the courtesy to discuss your concerns with you. Failure to return your call at all is inexcusable. Move on if at all possible or you will be facing lots of sleepless nights worrying that something needs attention and your little one is not getting it and could be getting worse instead of better.

 

[Printer]

A few years ago, I found it necessary to change CF Clinics. Health wise, It was a life saving move. Good luck

Bill

 

[emason]

I wanted to update!

His cough never turned croupy - but he continued to get worse. A couple days after this initial post I ended up at urgent care with him as his breathing while he was asleep was around 50 per minute. Urgent care diagnosed an ear infection and did a chest scan because they thought maybe pneumonia. Ultimately they said it was an "early" lung infection - although I'm not sure what that means. He ended up on a course of antibiotics and is fine now.

I reached out to his clinic after the DX from urgent care and the nurse said she'd talk to our pulm and get back to me. Never heard anything.

So I took everyone's advice and have begun the process of switching clinics! We are still finalizing everything but we should be going in to the new clinic next month and if it's a fit then we will be making the switch official. So far, after only one conversation , I feel 100% better about this new clinic. Thanks for all the advice!

 

[Ratatosk]

Glad to hear he's feeling better and you're switching clinics. With our old clinic, I would get so stressed out prior to and during appointments. Was such a relief when we opted to switch our primary care to our current clinic. BTW, I'm still waiting to hear back from the old one 10 years ago when I called about ds being exposed to whooping cough and what should we do. Fortunately, ds' current doctor called in a script for azithromycin immediately.

 

[emason]

I feel the same with our current clinic - just anxiety when trying to get a response to anything. And they've really treated me I'm such a way when I voice concerns that it leaves me wondering why my son is being followed at all if all they are going to do is ignore us.

The clinic I am switching him to had a reputation for being more aggressive with their crms cases. Had my son not been so ill over the last 10 months I would probably not mind the passive way that our first clinic treats us but I continue to feel like there is something going wrong him by the way he gets with each illness. Especially when some of them have also hit my 3 year old (who is only a carrier) and how quickly he has fought them off with no intervention. I can't shake the feeling that my son with crms has all the makings of a "the signs were there early" situation. I do think that it's safe to say he does not have a classic presentation but I do have many concerns that he will continue to have some mild symptoms that early acknowledgement of and preventative care may help him in the long run. If nothing else, better monitoring cannot hurt!

I feel very good about this switch and thank you all for helping me to feel validated in my concerns when our clinic didn't.

 

[emason]

Another update:

Went to the new clinic today and they feel he would benefit from cpt, abuterol and pulmicort. So we are going from zero treatments to now twice daily. The new pulmonologist believes that his constant respiratory illnesses are being compounded by inflammation and that with treatments we should see an improvement in his general health. Quite fittingly he also woke up with a runny nose and cough this morning so it was almost like an "aha" moment for me that the trend of him being sick over and over again is still continuing. I'm hopeful that this new clinic is truly taking him serious. It's a huge relief!

 

[Gammaw]

Amen! So glad they are being proactive and that you trusted your instincts and pushed!
Blessings!

 

[triples15]

Another update:

Went to the new clinic today and they feel he would benefit from cpt, abuterol and pulmicort. So we are going from zero treatments to now twice daily. The new pulmonologist believes that his constant respiratory illnesses are being compounded by inflammation and that with treatments we should see an improvement in his general health. Quite fittingly he also woke up with a runny nose and cough this morning so it was almost like an "aha" moment for me that the trend of him being sick over and over again is still continuing. I'm hopeful that this new clinic is truly taking him serious. It's a huge relief!

Yes! Great job mom! You are an amazing advocate for your little guy and he is so lucky to have you.

I'm so sorry that he isn't feeling well again, but at least you can rest assured that he is going to get the treatment he needs.

Take care, hope he's feeling better soon!

Autumn 34 w/cf

 

[Beccamom]

I am am so glad you switched clinics. As far as the culture and the symptoms coming up later that is exactly why the cultures are needed. My lungs are colonized with PA bacteria, but it does not always cause symptoms. What happens is if I catch a cold virus, then my body cannot fight both the new virus and the usual bacteria in my lungs. So I need antibiotics during every cold virus. The antibiotics do not fight the virus of course, but they hold back the bacteria allowing my body to have the ability to fight the virus. Knowing which bacteria your child cultures allows the correct antibiotic to be chosen that will work verses guessing. All cultures should be sent for sensitivities so you should know what antibiotic will work as soon as you need one.

Yes! Great job mom! You are an amazing advocate for your little guy and he is so lucky to have you.

I'm so sorry that he isn't feeling well again, but at least you can rest assured that he is going to get the treatment he needs.

Take care, hope he's feeling better soon!

Autumn 34 w/cf

 

[emason]

Thanks for that info Autumn! That's actually what I have been trying to understand about how cultures might be interpreted with symptoms. My son had another positive culture for A. Baumannii last month and his latest culture from a couple weeks ago is prelimanry for staph again. Our pulm decided it made sense to do a bronchoscopy since my son continues to have constant positive cultures so we are trying to get that scheduled. He thinks it's likely that it might be more related to reflux (as he's been having more and more reflux symptoms) , and that reflex is bringing up things from his gut which are showing on the throat cultures so the GI is also doing an endoscopy. I'm not exactly clear on what (if any) significance that would have. But he does think that given his history of respiratory illnesses and positive cultures, that we ought to find out what's going on. While they are at it, and since he will already be knocked out, they are going to do an infant PFT on him. Looks like we will have all the bases covered and a good baseline to work from moving forward.

FWIW, and because I was confused on the matter I asked him how they would have proceeded in the scenario that I described at the start of this thread and he did say that if they were treating my son when he's had those last few illnesses that led to respiratory involvement, they would have prescribed antibiotics specific to his cultures. We are currently on a healthy kick though (yay!) and so we haven't had to go that route as of yet.